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IBI Test 3 > B3.041 Prework Intro to Lymphoid Neoplasms > Flashcards

B3.041 Prework Intro to Lymphoid Neoplasms Flashcards

1
Q

where can lymphoid neoplasms be derived from?

A

precursor cells (B or T ALL)
mature B cells
mature T/MK cells

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2
Q

where can lymphoid neoplasms present?

A

primarily bone marrow/blood
primarily in lymph nodes
primarily in extranodal tissues

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3
Q

how are lymphoid neoplasms classified

A

cell of origin
development stage of transformed cell (precursor or mature)
clinical, immunophenotypic, genetic, and molecular findings
DOES NOT distinguish primarily blood/BM (leukemia) from primarily lymph node/tissue presentation (lymphoma)

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4
Q

same as chronic lymphocytic leukemia

A

small lymphocytic lymphoma

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5
Q

same as acute lymphoblastic leukemia

A

lymphoblastic lymphoma

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6
Q

what % of all lymphoid neoplasms involve B cells

A

80%

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7
Q

what % of all lymphoid neoplasms involve T/NK cells

A

20%

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8
Q

what type of lymphoma is classified neither as T or B cell?

A

Hodgkin

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9
Q

what are the 5 most common non-Hodgkin lymphomas?

A 
diffuse large B cell lymphoma
follicular lymphoma
MALT lymphoma
mature T cell lymphomas
CLL/SLL
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10
Q

which 3 mature B cell neoplasms occur in the BM/blood

A

CLL/SLL
hairy cell leukemia
Burkitt

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11
Q

which 5 mature B cell neoplasms occur in the lymph nodes

A 
CLL/SLL
follicular lymphoma
diffuse large B cell lymphoma
mantle cell lymphoma
Burkitt
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12
Q

which 2 mature B cell neoplasms occur extranodally

A

diffuse large B cell lymphoma

marginal zone lymphoma

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13
Q

which 2 mature T cell neoplasms occur in the BM/blood

A

adult T cell leukemia/lymphoma

mycosis fungoides/Sezary syndrome

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14
Q

which 3 mature T cell neoplasms occur in the lymph nodes

A

peripheral T cell lymphoma, unspec
anaplastic large cell lymphoma
adult T cell leukemia/lymphoma

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15
Q

which mature T cell neoplasm occurs extranodally

A

mycosis fungoides/ Sezary syndrome

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16
Q

what are some immunophenotypic markers of mature B neoplasms

A

CD19, CD20
monoclonal surface Ig (K or L restricted)
others disease based

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17
Q

what are some immunophenotypic markers of mature T neoplasms

A

CD2, CD3, CD5, CD7 (some may be absent)
CD4 and/or CD8 (variable, do NOT establish clonality)
others diseased based

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18
Q

genetic features of mature B neoplasms

A

monoclonal rearranged Ig genes
translocations involving chromosome 14 (IgH gene locus, constitutively expressed)
disease specific findings

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19
Q

genetic features of mature T neoplasms

A

monoclonal rearranged T-cell receptor genes (a/B or y/d)

disease specific findings

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20
Q

what are 2 different possible lymph node architectures of lymphoid neoplasms?

A

diffuse

nodular/follicular

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21
Q

distinguish between small and medium-large cell lymphoid neoplasms

A

small
-more indolent
-immunophenotype and genetics are critical for diagnosis and classification
medium-large
-tend to be more aggressive
-morphology supplemented by immunophenotype and genetics for diagnosis and classification

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22
Q

clinical features of chronic lymphocytic leukemia/ small lymphocytic lymphoma (CLL/SLL)

A 
older adults
most common leukemia in the US
variable anemia, thrombocytopenia
lymphadenopathy
immunologic abnormalities- autoimmune anemia or thrombocytopenia, monoclonal immunoglobulin (paraprotein)
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23
Q

morphology of CLL/SLL

A

CLL - small mature lymphocytes in blood, BM

SLL- diffuse infiltrates of small lymphocytes in lymph nodes

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24
Q

histologic features of CLL/SLL

A

smudge like cells w no cytoplasm

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25
Q

immunophenotype of CLL/SLL

A

CD19+
CD20+
CD5+
sIg+ (clonal)

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26
Q

genetics of unmutated CLL/SLL (U-CLL/SLL)

A

no somatic hypermutation in IgV
40-50% of cases
unfavorable

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27
Q

genetics of mutated CLL/SLL (M-CLL/SLL)

A

somatic hypermutation in IgV
50-60% of cases
favorable

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28
Q

course and prognosis of CLL/SLL

A 
median survival 6 y
-worse for U-CLL/SLL (3) than M-CLL/SLL (7)
prolymphocytic transformation - 15-30%
-mean survival <2 y
Richter transformation - 5-10%
-large cell lymphoma
-increased lymphadenopathy
-mean survival <1 y
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29
Q

clinical features of hairy cell leukemia

A

middle aged and older adults
rare
splenomegaly, hepatomegaly
pancytopenia, infections

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30
Q

morphology/histological features of hairy cell leukemia

A

villous projections

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31
Q

immunophenotype of hairy cell leukemia

A 
CD19+
CD20+
sIg+ (clonal)
CD11c+
CD25+
CD103+ **
annexin A1 **
**very specific
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32
Q

genetics of hairy cell leukemia

A

BRAF mutation

also seen in other cancers, targeted therapies exist

33
Q

course and prognosis of hairy cell leukemia

A

indolent

34
Q

clinical features of follicular lymphoma

A

middle aged and older adults
common (2nd most common non Hodgkin)
lymphadenopathy
hepatosplenomegaly

35
Q

morphology of follicular lymphoma

A

nodular infiltrate of small cleaved lymphocytes in lymph nodes
diminished mantle zone
absent tangible body macrophages

36
Q

immunophenotype of follicular lymphoma

A

CD19+
CD20+
CD10+
sIg+ (clonal)

37
Q

genetics of follicular lymphoma

A

t(14;18)

overexpression of BCL2 which prevents apoptosis

38
Q

course and prognosis of follicular lymphoma

A

indolent

39
Q

clinical features of mantle cell lymphoma

A

middle aged and older adults
uncommon
lymphadenopathy
hepatosplenomegaly (often advanced stages)

40
Q

morphology of mantle cell lymphoma

A

nodular or diffuse infiltrate of small lymphocytes in lymph nodes

41
Q

immunophenotype of mantle cell lymphoma

A 
CD19+
CD20+
CD5+
sIg+ (clonal)
above same as CLL/SLL
cyclin D1+
42
Q

genetics of mantle cell lymphoma

A

t(11;14) overexpression of cyclin D1

involved in cell division (oncogene)

43
Q

course and prognosis of mantle cell lymphoma

A

poor prognosis
median survival 3-4 y
unusually aggressive for small cell

44
Q

clinical features of marginal zone lymphoma

A

extranodal - GI tract, lung, skin, eye, head & neck
association with immune reaction and chronic infection (H. pylori)
-can sometimes be treated early by treating underlying infection

45
Q

morphology of marginal zone lymphoma

A

infiltrate of lymphoid cells into epithelium and expanded marginal zone

46
Q

immunophenotype of marginal zone lymphoma

A

CD19+
CD20+
sIg+ (clonal)

47
Q

course and prognosis of marginal zone lymphoma

A

indolent

48
Q

clinical features of diffuse large B cell lymphoma

A 
middle aged and older adults
most common lymphoma
lymphadenopathy
hepatosplenomegaly
frequently extranodal
49
Q

morphology of diffuse large B cell lymphoma

A

diffuse infiltrate of large lymphocytes in lymph nodes

50
Q

immunophenotype of diffuse large B cell lymphoma

A 
CD19+
CD20+
CD10+/-
sIg+ (clonal)
some types associated with EBV or HHV8
51
Q

genetics of diffuse large B cell lymphoma

A

may be positive for MYC translocation (most aggressive)

52
Q

course and prognosis of diffuse large B cell lymphoma

A

aggressive

53
Q

clinical features of Burkitt

A 
rapidly progressive tumor mass
-extremely high growth fraction
-tumor lysis syndrome common w treatment
-fast, but responsive to treatment
endemic form in Africa
-primarily kids
- >95% associated with EBV
-mandible, abdomen
sporadic form in US
-involved kids and adults
-15-20% associated with EBV
-abdomen
HIV associated form
-worst response to therapy
-25% associated with EBV
54
Q

morphology of Burkitt

A

diffuse infiltrate of medium sized lymphocytes in lymph nodes
high mitotic rate
tingible body macrophages (starry sky)

55
Q

immunophenotype of Burkitt

A 
CD19+
CD20+
CD10+
sIg+ (clonal)
TdT- (can look like ALL, but ALL is TdT+)
56
Q

genetics of Burkitt

A

t(8;14) [variants t(2;8) or t(8;22)] MYC oncogene translocated to IgH locus and constitutively expressed

57
Q

course and prognosis of Burkitt

A

highly aggressive course but high cure rates (60-90%) w chemotherapy

58
Q

clinical features of mycosis fungoides/sezary syndrome

A

older adults
cutaneous patch, plaque, nodule stages
lymphadenopathy unusual

59
Q

morphology of MF/SS

A

MF- dermal infiltrate of atypical lymphocytes with epidermotropism and Pautrier microabcesses
SS- circulating lymphoid cells with cerebriform nuclei and erythroderma

60
Q

immunophenotype of MF/SS

A

CD4+ T cells

61
Q

course and prognosis of MF/SS

A

indolent
chronic course with 90% 5 year survival
SS more aggressive form

62
Q

clinical features of adult T cell leukemia/lymphoma

A

endemic in Japan, Caribbean, West Africa
middle aged to older adults
caused nu HTLV-1 infection
generalized lymphadenopathy, skin lesion, hepatosplenomegaly, lymphocytosis, and hypercalcemia

63
Q

course and prognosis of adult T cell leukemia/lymphoma

A

poor prognosis especially in leukemic phase

64
Q

clinical features of anaplastic large cell lymphoma

A

lymphadenopathy

65
Q

immunphenotype of anaplastic large cell lymphoma

A

CD30+

66
Q

genetics of anaplastic large cell lymphoma

A

t(2;5) rearrangement of ALK

67
Q

course and prognosis of anaplastic large cell lymphoma

A

poor prognosis if ALK-

68
Q

clinical features of peripheral T-cell lymphoma

A 
lymphadenopathy
fever
pruitis
weight loss
eosinophilia (secretion of IL-5)
69
Q

course and prognosis of peripheral T cell lymphoma

A

poor prognosis with 20-30% 5 year survival rate

70
Q

clinical features of Hodgkin

A

bimodal age distribution (15-35 and >50)
most common malignancy in 10-30 y age group in US
lymphadenopathy, splenomegaly
constitutional (“B”) symptoms- fever, night sweats, weight loss
immune dysfunction
possible relation to EBV

71
Q

morphology of Hodgkin

A

classical - Reed-Sternberg cells in a polymorphous cellular background
nodular lymphocyte predominance - no Reed-Sternberg cells; large B cells in a background of small lymphocytes

72
Q

describe Reed-Sternberg cells

A

extremely large
2 nuclei
large nucleous with clearing around it

73
Q

what are some features that make Hodgkin unique from non-hodgkin

A 
heterogeneous population
reed-Sternberg
primary extranodal presentation rare
often localized to a single nodal group
spread along contiguous nodal groups
tonsils and adenoids rarely involved
young adults and elderly
good prognosis
74
Q

immunophenotype of Hodgkin

A 
reed Sternberg cells
CD15+
CD30+
CD20+/-
negative for most other T cell markers
75
Q

genetics of Hodgkin

A

mutate germinal center B cells that escape apoptotic death

76
Q

course and prognosis

A

excelled prognosis in early stage (90% cure rate)

risk of secondary malignancy in long term survivors (due to chemo and radiation)

77
Q

non-neoplastic proliferations of lymphocytes

A

viral infections
acute bacterial infections
chronic bacterial infections
atypical lymphocytes

78
Q

non-neoplastic lymphadenopathy

A

follicular hyperplasia- infection, autoimmune disorders, drug reaction
paracortical hyperplasia- viral infections, drug reactions
sinus histiocytosis- lymph nodes draining infections of cancer

IBI Test 3 (19 decks)

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